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Ali M Allahdina, Katherine G Chen, Jason A Alvarez, Wai T Wong, Emily Y Chew, Catherine A Cukras
PURPOSE: To characterize functional and structural changes in hydroxychloroquine (HCQ) retinal toxicity after drug cessation. METHODS: Twenty-two patients (91% female; mean age 58.7 ± 11.4 years; mean duration of HCQ treatment 161.1 ± 90 months; mean dose 5.9 ± 1.9 mg/kg) with detected HCQ retinopathy were monitored for 6 months to 82 months after HCQ cessation with multimodal imaging including spectral domain optical coherence tomography and fundus autofluorescence imaging at 488 nm (standard) and 787 nm (near-infrared autofluorescence)...
February 5, 2019: Retina
Teemu T Turunen, Ari Koskelainen
Light regulates cGMP concentration in the photoreceptor cytoplasm by activating phosphodiesterase (PDE) molecules through a G-protein signalling cascade. Spontaneous PDE activity is present in rod outer segments even in darkness. This basal PDE activity (βdark ) has not been determined in wild type mammalian photoreceptor cells although it plays a key role in setting the sensitivity and recovery kinetics of rod responses. We present a novel method for determination of βdark using local electroretinography (LERG) from isolated mouse retinas...
February 4, 2019: Scientific Reports
Anna Peresypkina, Anton Pazhinsky, Mikhail Pokrovskii, Evgenya Beskhmelnitsyna, Anna Pobeda, Mikhail Korokin
An important task of pharmacology and ophtalmology is to find specific and highly effective agents for correcting retinal ischemia. The objective of this study is to increase the effectiveness of pharmacological correction of retinal ischemia by using new 3-hydroxypyridine derivative⁻l-isomer of ethylmethylhydroxypyridine malate. A modification to the retinal ischemia-reperfusion model was used, in which an increase in intraocular pressure is carried out by mechanical pressure (110 mmHg) to the front chamber of the eye for 30 min...
February 3, 2019: Antioxidants (Basel, Switzerland)
Yuyi You, Ling Zhu, Ting Zhang, Ting Shen, Ariadna Fontes, Con Yiannikas, John Parratt, Joshua Barton, Angela Schulz, Vivek Gupta, Michael H Barnett, Clare L Fraser, Mark Gillies, Stuart L Graham, Alexander Klistorner
PURPOSE: To compare functional and structural changes in the retina in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS). DESIGN: Cross-sectional study; biochemical study of human retinas. PARTICIPANTS: A total of 181 participants, including 22 consecutive patients (44 eyes) with NMOSD, 131 patients (262 eyes) with multiple sclerosis (MS), and 28 normal subjects (56 eyes)...
January 31, 2019: Ophthalmology
Sarah Koll-Hampp, Andra-Elena Enache, Elena Fenollosa-Romero, Yu-Mei Chang, Claudia Busse, James Oliver, Charlotte Dawson, Màrian Matas Riera
OBJECTIVE: To describe the visual outcome following phacoemulsification in English Cocker Spaniels (ECS) affected by cataracts and suspected progressive retinal atrophy (PRA). ANIMALS STUDIED: Fifty-four client-owned dogs. PROCEDURES: A multicenter, retrospective study was performed including ECS with suspected PRA which underwent phacoemulsification. PRA was suspected on ophthalmic examination before and after surgery, and/or after electroretinography (ERG) was performed...
January 31, 2019: Veterinary Ophthalmology
Kenji Ozawa, Kiyofumi Mochizuki, Yusuke Manabe, Nobuaki Yoshikura, Takayoshi Shimohata, Ichizo Nishino, Yu-Ichi Goto
PURPOSE: Mitochondrial encephalopathy with lactic acid and stroke-like episodes (MELAS) is caused by mutations in the mitochondrial DNA. Approximately 80% of MELAS patients have an A > G transition mutation at nucleotide pair 3243 in the mitochondrial DNA, m.3243A > G. There are also MELAS patients with a one-base deletion at nucleotide pair 3271 in the mitochondrial DNA, m.3271delT, but these cases are very rare. We report a case of MELAS with the m.3271delT and describe the retinal structure and electrophysiological alterations...
January 30, 2019: Documenta Ophthalmologica. Advances in Ophthalmology
Reiko Tanabu, Kota Sato, Natsuki Monai, Kodai Yamauchi, Takayuki Gonome, Yuting Xie, Shizuka Takahashi, Sei-Ichi Ishiguro, Mitsuru Nakazawa
PURPOSE: Mutations of the gene encoding RPE65 cause Leber congenital amaurosis (LCA) retinitis pigmentosa (RP). The optical coherence tomography (OCT) is increasingly utilized to noninvasively evaluate various types of retinal diseases, including RP. The present study was conducted to characterize the OCT findings of the RPE65-/- mice-an animal model of LCA and RP-in relation to the morphological features based on histological and electron microscopic findings as well as electroretinography (ERG) features...
2019: PloS One
Le-Tien Lin, Jiann-Torng Chen, Ming-Cheng Tai, Yi-Hao Chen, Ching-Long Chen, Shu-I Pao, Cherng Ru Hsu, Chang-Min Liang
Ischemia-reperfusion (I/R) injury is associated with numerous retinal diseases, such as diabetic retinopathy, acute glaucoma, and other vascular retinopathies. Hypercapnic acidosis (HCA) has a protective effect on lung, myocardial, and central nervous system ischemic injury models. However, no study has evaluated its protective effects in an experimental retinal I/R injury model. In this study, retinal I/R injury was induced in Sprague Dawley rats by elevating the intraocular pressure to 110 mmHg for 60 minutes...
2019: PloS One
Yunkao Zeng, Dan Cao, Honghua Yu, Dawei Yang, Xuenan Zhuang, Yunyan Hu, Juan Li, Jing Yang, Qiaowei Wu, Baoyi Liu, Liang Zhang
AIMS: To investigate the function and the corresponding neurovascular structures in patients with diabetes without clinically detectable retinopathy. METHODS : Sixty-six patients with type 2 diabetes without retinopathy (NDR) and 62 healthy controls were recruited. The 16 and 32 Tds flicker electroretinography (ERG) was performed using a mydriasis-free, full-field flicker ERG recording device (RETeval). The vessel density (VD) of superficial capillary plexus (SCP) and deep capillary plexus (DCP), FD300 and ganglion cell complex (GCC) thickness in the macula were quantified using optical coherence tomography angiography (OCTA)...
January 23, 2019: British Journal of Ophthalmology
Yusaku Katada, Kenta Kobayashi, Kazuo Tsubota, Toshihide Kurihara
Purpose: The most common virus vector used in gene therapy research for ophthalmologic diseases is the adeno-associated virus (AAV) vector, which has been used successfully in a number of preclinical and clinical studies. It is important to evaluate novel AAV vectors in animal models for application of clinical gene therapy. The AAV-DJ (type 2/type 8/type 9 chimera) was engineered from shuffling eight different wild-type native viruses. In this study, we investigated the efficiency of gene transfer by AAV-DJ injections into the retina...
2019: PeerJ
Fan-Li Lin, Yu-Wen Cheng, Min Yu, Jau-Der Ho, Yu-Cheng Kuo, George C Y Chiou, Hung-Ming Chang, Tzong-Huei Lee, George Hsiao
BACKGROUND: Elevated intraocular pressure (IOP) is a major risk factor for glaucoma that has been found to induce matrix metalloproteinase-9 (MMP-9) activation and result in eventual retinal dysfunction. Proinflammatory cytokines such as monocyte chemoattractant protein-1 (MCP-1) and interleukin-1β (IL-1β) were also found to be involved in disease progression by mediating MMP-9 production. We previously reported that fungal derivative theissenolactone C (LC53) could exert ocular protective effects by suppressing neuroinflammation in experimental uveitis...
November 7, 2018: Phytomedicine: International Journal of Phytotherapy and Phytopharmacology
Jun Ho Yoo, Cheolmin Yun, Jaeryung Oh, Seong-Woo Kim
AIM: To investigate whether the response of a central hexagonal element corresponding to the macular area in conventional multifocal electroretinography (mfERG) tests was the same as that of experimental mfERG using single central hexagonal element stimulation. METHODS: Prospective, observational study. Thirty healthy subjects were included in this study. mfERG recordings were performed according to two protocols: stimulus with 37 hexagonal elements (protocol 1), and stimulus with a single central element created by deactivating the other 36 hexagonal elements (protocol 2)...
2019: International Journal of Ophthalmology
Ariana M Levin, Jasmine H Francis, Molly McFadden, Scott Brodie, Y Pierre Gobin, David H Abramson
IMPORTANCE: Predictions of visual outcomes are useful in clinical and family decisions regarding treatment for retinoblastoma. Very little has been published on the association of post-treatment visual acuity with pre-treatment electroretinography (ERG), which can be performed on infants too young to reliably quantify visual acuity. OBJECTIVE: To report associations of pre-treatment ERG with post-treatment visual acuity in eyes with advanced retinoblastoma treated with ophthalmic artery chemosurgery (OAC)...
2019: PloS One
Jun Chen, Rachel R Caspi
Non-infections uveitis in humans is an autoimmune disease of the retina and uvea that can be blinding if untreated. Its laboratory equivalent is experimental autoimmune uveitis (EAU) induced in susceptible rodents by immunization with retinal antigens and described elsewhere in this series (Agarwal et al., Methods Mol Biol, 900:443-469, 2012). Evaluation and quantitation of the disease is usually performed by fundus examination and/or histopathology, which provide limited information on structural and no information on functional changes as disease progresses...
2019: Methods in Molecular Biology
Guoxin Ying, Jeanne M Frederick, Wolfgang Baehr
Centrins (CETN1-4) are ubiquitous and conserved EF-hand family Ca2+ -binding proteins associated with the centrosome, basal body, and transition zone. Deletion of CETN1 or CETN2 in mice causes male infertility or dysosmia, respectively, without affecting photoreceptor function. However, it remains unclear to what extent centrins are redundant with each other in photoreceptors. Here, to explore centrin redundancy, we generated Cetn3 GT/GT single-knockout and Cetn2 -/- ; Cetn3 GT/GT double-knockout mice. Whereas the Cetn3 deletion alone did not affect photoreceptor function, simultaneous ablation of Cetn2 and Cetn3 resulted in attenuated scotopic and photopic electroretinography (ERG) responses in mice at three months of age, with nearly complete retina degeneration at one year...
January 15, 2019: Journal of Biological Chemistry
Fen Tang, Michelle E LeBlanc, Weiwen Wang, Dan Liang, Ping Chen, Tsung-Han Chou, Hong Tian, Wei Li
Retinopathy of prematurity (ROP) with pathological retinal neovascularization is the most common cause of blindness in children. ROP is currently treated with laser therapy or cryotherapy, both of which may adversely affect the peripheral vision with limited efficacy. Owing to the susceptibility of the developing retina and vasculatures to pharmacological intervention, there is currently no approved drug therapy for ROP in preterm infants. Secretogranin III (Scg3) was recently discovered as a highly disease-restricted angiogenic factor, and a Scg3-neutralizing monoclonal antibody (mAb) was reported with high efficacy to alleviate oxygen-induced retinopathy (OIR) in mice, a surrogate model of ROP...
January 14, 2019: Angiogenesis
Ying Chen, Zilin Chen, Wai Po Chong, Sihan Wu, Weiwei Wang, Hongyan Zhou, Jun Chen
Experimental autoimmune uveitis (EAU) represents autoimmune uveitis in human, among which B10.RIII and C57BL/6 are the frequently used strains in mice, but to day no study has been reported to compare EAU disease between the two strains. Here we compared the differences in morphology, pathology, visual function of the retinal inflammation and Th1/Th17 immune responses in the EAU models induced by the interphotoreceptor retinoid binding protein (IRBP) between the B10.RIII and C57BL/6 strains, using fundus and histological examinations, optical coherence tomography, electroretinography and immunoassays...
January 12, 2019: Current Molecular Medicine
Andrea Cusumano, Benedetto Falsini, Emiliano Giardina, Raffaella Cascella, Jacopo Sebastiani, John Marshall
BACKGROUND: Based on phenotypic similarities between age-related macular degeneration and the autosomal disorder Doyne honeycomb retinal dystrophy, we report on a single nanolaser treatment of a patient with genotype Doyne honeycomb retinal dystrophy confirmation and evidence of disease progression over 12 months. The case study is the first report of short-term results of subthreshold nanolaser treatment in a patient with Doyne honeycomb retinal dystrophy. CASE PRESENTATION: A 43-year-old Caucasian man with moderate loss of visual acuity in his left eye (20/40) and normal visual acuity in his right eye (20/20), with clinical Doyne honeycomb retinal dystrophy diagnosis and genetic confirmation of the common heterozygous mutation (EFEMP1) by genetic testing, underwent nanopulse subthreshold laser treatment in his left eye...
January 10, 2019: Journal of Medical Case Reports
Bincui Cai, Zhiqing Li, Shuo Sun, Linni Wang, Lu Chen, Jin Yang, Xiaorong Li
BACKGROUND: To clarify the diagnosis of a Chinese patient with novel double heterozygous in the NR2E3 and OPN1LW genes and describe the clinical features. MATERIALS AND METHODS: A 47-year-old man presented with an 8-year history of decreased vision and poor night vision. Based on his clinical phenotype, we focused on 36 genes associated with these characteristics. Possible pathogenic mutation sites were screened by next-generation sequencing (NGS), which showed novel mutations in the NR2E3 and OPN1LW genes...
January 7, 2019: Ophthalmic Genetics
Arif O Khan, Ibraheem A El-Ghrably
PURPOSE: To describe and distinguish overlapping retinal phenotypes in a consanguineous family harboring mutations in CRB1 and RS1, two different genes that are associated with splitting of the central neurosensory retina. METHODS: Retrospective case series. RESULTS: Three siblings and their father had decreased vision from early childhood, but the father was unavailable for clinical examination. The proband, an 11-year old boy, had clinical features classic for CRB1-retinopathy (nummular pigmentary degeneration, relatively plump vessels, thickened retinal by optical coherence tomography with cystoid splitting in the central macula)...
January 4, 2019: Ophthalmic Genetics
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